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Congenital Defects & Pediatrics IA > TGA- Topic 15 > Flashcards

TGA- Topic 15 Flashcards

1
Q

TGA/TGV

A

Discordant ventricular-arterial relationship

Malformation in which the two great arteries carrying blood away from the heart are transposed or reversed

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2
Q

The LV –>

A

PA

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3
Q

The RV –>

A

Aorta

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4
Q

What’s the main classification of CHD?

A

Acyanotic

Cyanotic

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5
Q

Two types of Acyanotic CHD

A

Increased pulmonary blood flow

Obstruction to blood flow from ventricles

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6
Q

Two types of Cyanotic CHD

A

Decreased blood flow

Mixed blood flow

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7
Q

Acyanotic: Increased Pulmonary Blood Flow

A

ASD
VSD
PDA
AV Canal

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8
Q

Acyanotic: Obstruction to blood flow from ventricles

A

Coarc of aorta
Aortic stenosis
Pulmonic stenosis

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9
Q

Cyanotic: Decreased pulmonary blood flow

A

TOF

Tricuspid Atresia

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10
Q

Cyanotic: Mixed Blood Flow

A

TGA
TAPVR
TA
HLHS

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11
Q

TGA is compatible with life unless what happens?

A

Some communication exists between the two separate circulatory systems.

Frequently patients with TGA have ASD’s or VSD’s

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12
Q

What are the two parallel circulations in TGA?

A

Body- RA - RV- Ao-Body

Lungs- LA- LV- PA- Lungs

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13
Q

What type of mixing in TGA?

A

Poor mixing

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14
Q

What are some symptoms of TGA?

A 
Hypoxia & Acidemia
Hyperventilation
Increased pulmonary flow
CHF
Myocardial depression
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15
Q

What is the most common cyanotic congenital heart lesion presenting in the neonate?

A

TGA

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16
Q

What percent of cases are TGA?

A

5%

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17
Q

TGA: Gender

A

More common in males, with a ratio of about 3:1

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18
Q

What are some maternal factors that are associated with an increased of TGA?

A

Rubella or other virus during pregnancy
Alcoholism
Maternal age over 40
Diabetes

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19
Q

TGA is a defect of the …..

A

Bulbus cordis

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20
Q

TGA: After outflow tract septation development beings then improper spiraling of _____________. This leads to…..

A

the aorticopulmonary septum
This leads to congenital disruption in pulmonary and systemic circulations (sounds a bit like the TOF defect development)

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21
Q

Truncus Arteriosus becomes…..

A

Aorta

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22
Q

Conus Cordis becomes… What is this created by?

A

Pulmonary Artery; created by a septum that forms in the outflow tract from these swellings

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23
Q

What day is outflow tract septation?

A

Day 29

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24
Q

As is with TAPVR, without intervention infants with TGA will die within how long?

A

Their first year of life

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25
What are the two anatomical classifications of TGA?
D-Transposition (Right) | L-Transposition (Left)
26
In what percent of patients is the aorta anterior and to the right of hte pulmonary artery?
60% | Dextro-transposition of the great arteries (d-TGA)
27
What is called when the aorta is anterior and to the left of the pulmonary artery?
(levo-transposition of the great arteries (l-TGA)
28
What is the only distinguishing characteristic that defines TGA?
Discordant ventriculo-arterial connection
29
What are presentations common to TGA?
1. TGA w/ intact ventricular septum 2. TGA w/ VSD 3. TGA w/ VSD and LVOT obstruction 4. TGA w/ VSD and Pulmonary vascular obstructive disease
30
TGA first described over 200 years ago tx was not available until:
1. development of surgical atrial septectomy in the 1950's | Balloon atrial septostomy in the 1960's.
31
Palliative therapies followed by:
``` Physiological procedures (atrial switch operation) Anatomic repair (arterial switch operation) ```
32
What is the survival rate for infants with TGA?
Greater than 90%
33
What is the initial treatment consist of?
Ductal patency with continuous IV prostaglandin E1 infusion (PGE1)
34
What does continuous IV prostaglandin E1 infusion PGE1 result in?
Increased pulmonary blood flow Increased left atrial pressure Promotes L --> R shunting at the atrial level (decreased cyanosis) Impt in patients with severe LVOT (PDA will not help when the defect has intact septum's and separate circulations)
35
Why might an arterial switch operation not be feasible in TGA w/ IVS and LVOT obstruction?
Pulmonary (LVOT) stenosis or atresia
36
If the VSD is nonrestrictive and not too remote from the aorta, what repair could be possible?
Rastelli intracardiac repair
37
Rastelli Procedure
conduit from the RV to the PA, delaying repair *placing an aorto-pulmonary shunt during the newborn period may be necessary to establish adequate pulmonary blood flow while waiting (i.e. central shunt)
38
What are the two types of atrial switch procedures?
Mustard | Senning
39
What are the two types of arterial switches?
Jatene | Le COmpte
40
Mustard procedure- atrial baffle
Restores circulation, but reverses the direction of the blood flow in the heart Blood is pumped to the lungs via the LV and disseminated throughout the body via the RV But the right ventricle is not the optimal shape to support the high pressure work performed in an ormal heart by the LV
41
What type of baffle does the mustard procedure use?
Pericardial tissue baffle
42
Senning Procedure
A baffle is created within the atria that redirects the deoxygenated caval blood to the mitral valve and the oxygenated pulmonary venous blood to the tricuspid valve The anatomic LV continues to act as the pulmonary pump and the anatomic RV acts as the systemic pump
43
What is the dfiference beween the Mustard procedure and Senning procedure?
Identical except that the baffle is constructed from atrial tissue in the Senning and from pericardium in the Mustard
44
What is the standard therapy for transposition?
The neonatal arterial switch procedure; by the 1980's it was adopted
45
The Mustard procedure was replaced in the late 1970's by what procedure?
Jatene procedure (arterial switch)
46
Jatene Procedure
Native arteries are switched back to normal flow, so that the RV would be connected to the pulmonary artery and the LV would be connected to the aorta
47
Why was the Jatene procedure not possible prior to 1975?
because of the difficulty with re-implanting coronary arteries which perfuse myocardium
48
CPB Considerations TGA: Cannulation
Arterial: Aortic Venous: Single Atrial (bicaval if 4+ kg)
49
CPB Considerations TGA: Hypothermia
DHCA/Low flow w/ HCA
50
CPB Considerations TGA: Cardioplegia
Antegrade, Retrograe, Ostial (multiple dosing)
51
What type of weight do TGA kids have?
These children are larger weight; around 3 kg
52
Is complete correction or palliation more common to tx TGA?
Complete correction
53
is the post-procedure for TGA open or close?
Open chest-post procedure (silastic patch)
54
What is the length of the procedure to correct TGA?
Longer procedure, technically difficult
55
Truncus Arteriosus AKA
persistent truncus arteriosus
56
Truncus Arteriosus
a rare type of congenital heart disease comes out of hte right and left ventricles, instead of the normal two (pulmonary artery and aorta)
57
In TA is untreated what two problems occur?
1. Too much pulmonary flow 2. The blood vessels to the lungs become permanently damaged. Pulmonary hyptertension develops; the major problem in Truncus is that the lungs are flooded w/ blood and the heart muscle is overloaded
58
TA Symptoms
``` Bluish skin (cyanosis) Delayed growth or growth failure Fatigue Lethargy Poor feeding Rapid breathing (tachypnea) Shortness of breath (dyspnea) Widening of the finger tips (clubbing) ```
59
What causes TA?
Failed septation of the embryonic truncus arteriosus. Aortiopulmonary and interventricular defects are believed to represent an abnormality of conotruncal septation. Because of the common trunk originates from both the LV and RV, and PAs arise directly from the common trunk, a PDA is not required to support the fetal circulation
60
What divides the truncus arterosus and bulbus cordis?
Aortico-pulmonary septum
61
Truncus arteriosus gives rise to what?
The ascending aorta and the pulmonary trunk
62
What does the bulbus cordis give rise to?
The smooth parts (outflow tract) of the LV and RV
63
TA: Type I
Truncus --> One pulmonary artery --> Two lateral pulmonary arteries Origin of a single pulmonary trunk from the left lateral aspect of hte common trunk, with branching of hte left and right pulmonary arteries form the pulmonary trunk
64
TA: Type II
Truncus --> two poster/posteriolateral pulmonary arteries separate but proximate origins of hte left and right pulmonary arterial branches from the posterolateral aspect of hte common arterial trunk
65
TA: Type III
Truncus --> two lateral pulmonary arteries Branch pulmonary arteries oringinate independently from the common arterial trunk or aortic arch (most often from the left and right lateral aspects of hte trunk) This occasionally occurs with origin of one pulmonary artery from the underside of hte oartic arch, usually from a ductus arteriosus
66
Palliative Surgery for TA
PA Banding; left anterior thoracotomy approach through the second or third intercostal space gives excellend exposure for isolated PAB Extracardiac procedure -no CPB
67
What is the treatment of choice for TA?
Complete repair and usually a modification of a Rastelli procedure. A Rastelli procedure connects the RV-PA. This tube is usually a homograft (made a human vadaver tissue) During the Rastelli procedure, the VSD is closed with a Core-Tex patch so that hte oarta arises solely from the left ventricle
68
CPB TA: Incision
Median Sternotomy
69
CPB TA: Cannulation
Arterial - Aortic | Venous-Single Atrial cannula
70
CPB TA: XC
moderate lenght
71
CPB TA: hypothermia
mild to moderate period DHCA
72
CPB TA CPG
antegrade possibly retorgrade.

Congenital Defects & Pediatrics IA (17 decks)

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